What is juvenile xanthogranuloma (JXG)?

Updated: Sep 16, 2020
  • Author: Cameron K Tebbi, MD; Chief Editor: Vikramjit S Kanwar, MBBS, MBA, MRCP(UK), FAAP  more...
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Juvenile xanthogranuloma 

Juvenile xanthogranuloma (JXG) is a self-limited dermatologic disease of infancy with rare systemic manifestation. The dermatologic findings often include multiple yellow-to-pink, firm, slightly raised cutaneous papulonodules, which usually appear in the head and neck region. The nodules often measure several millimeters in diameter, but a macronodular variant with lesions that measure several centimeters has also been described. Lesions can be observed in the deep soft tissues or organs. [65, 66, 67] The condition usually presents at birth but can be found during infancy. Similar lesions may also be seen in children and adults.

In histologic evaluation, the lesions are well circumscribed and consist of an accumulation of histiocytic cells with giant cells and spindle cells. Immunohistochemical studies usually reveal positivity for factor XIIIa, fascin CD68, and peanut agglutinin lectin. Results for S-100 protein is often, but not exclusively, negative.

The course of JXG is usually marked by spontaneous resolution of the lesions. Systemic forms of JXG that involve the CNS can be devastating. Although no treatment is usually necessary, chemotherapy may be required to manage systemic forms of the disease.

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